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Research ArticleArticle

Observational Study of Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis

ARIANE L. HERRICK, MARK LUNT, NINA WHIDBY, HOLLY ENNIS, ALAN SILMAN, NEIL McHUGH and CHRISTOPHER P. DENTON
The Journal of Rheumatology January 2010, 37 (1) 116-124; DOI: https://doi.org/10.3899/jrheum.090668
ARIANE L. HERRICK
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  • For correspondence: ariane.herrick@manchester.ac.uk
MARK LUNT
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NINA WHIDBY
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HOLLY ENNIS
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ALAN SILMAN
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NEIL McHUGH
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CHRISTOPHER P. DENTON
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    Figure 1.

    Completion of 3-year followup, by year.

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    Figure 2.

    Modified Rodnan skin score (mRSS) during followup. Shaded box: interquartile range; light bar: median; whiskers: the most extreme observations.

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    Figure 3.

    Modified Rodnan skin score (mRSS) during followup, by treatment protocol. Light bars show medians; shaded boxes show interquartile range. The whiskers show the most extreme observation less than 1.5 times the length of the shaded boxes. Dots show the individual observations beyond the whiskers. Protocol 1. IV cyclophosphamide followed by mycophenolate mofetil (MMF); Protocol 2. Antithymocyte globulin followed by MMF; Protocol 3. MMF alone; Protocol 4. No disease-modifying treatment; Protocol 5. Other immunosuppressant treatment.

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    Table 1.

    Baseline characteristics and antibody status of patients with systemic sclerosis by treatment group. Values are median (IQR) unless otherwise indicated.

    CharacteristicProtocol 1 n = 29Protocol 2 n = 25Protocol 3 n = 61Protocol 4 n = 19Protocol 5 n = 13p**Total (overall) n = 147
    Age, yrs55.1 (49.9–64.1)52.7 (45.7–57.8)54.4 (43.0–61.3)55.6 (50.1–67.2)40.9 (32.3–51.5)0.0153.1 (43.3–61)
    Women (%)18 (62)20 (80)44 (72)13 (68)8 (62)0.60103 (70)
    Antitopoisomerase (anti-Scl-70) no. (%)*8 (33)5 (20)14 (24)6 (33)4 (33)0.7237 (27)
    Anti-RNA polymerase III, no. (%)*1 (8)1 (4)9 (22)1 (14)0 (0)0.1513 (12)
    On corticosteroids, no. (%)9 (31)17 (68)16 (26)7 (37)8 (62)0.00257 (39)1
    Pulmonary hypertension, no. (%)6 (21)0 (0)0 (0)2 (11)0 (0)0.0018 (6)
    Pulmonary fibrosis, no. (%)16 (57)7 (28)11 (18)6 (32)3 (23)0.00843 (30)
    Cardiac involvement, no. (%)8 (28)1 (4)3 (5)2 (11)1 (8)0.02415 (10)
    mRSS, (0–51)24 (20.5–30)32 (30–41)23.5 (18–30)20.5 (18–22)21 (13–27)0.000124 (19–32)
    HAQ-DI score, 0–31.19 (0.56–2.00)1.63 (1.38–2.50)1.50 (0.75–2.13)1.00 (0.25–2.00)1.50 (0.00–2.63)0.411.50 (0.75–2.13)
    Functional Questionnaire, 0–3311.5 (3–18)18 (10–21)14.5 (7–21)10 (1–23)10 (0–11)0.1013.5 (6–19)
    Hemoglobin, g/l127 (114–134)117 (114–130)124 (116–137)123 (112–131)121 (114–134)0.60123 (114–134)2
    Platelets × 109/l312 (257–350)324 (270–414)329 (297–399)370 (284–441)370 (305–432)0.29328 (278–403)
    ESR, mm/h22 (7–37)21 (8.5–36)22 (10–34.5)28 (13.5–36)13 (9–18)0.7222 (10–36)
    Plasma creatinine, μmol/l70 (57–87)68 (64–82)70 (63–83)78 (72–104)63.5 (58–72)0.06771 (63–84)
    Pulmonary function tests
      FVC, % predicted76.0 (64.0–91.0)93.3 (86.2–101.5)87.8 (70.1–97.0)88.7 (81.0–97.0)84.7 (89.9–100.5)0.1687.7 (70.4–97.0)
      DLCO, % predicted58.5 (45.0–79.0)76.1 (64.3–81.6)71.5 (54.0–87.0)69.4 (48.6–85.0)81.0 (65.5–91.4)0.09171.0 (54.0–84.0)
    • ↵* Percentages related to the numbers tested.

    • ↵** Significance p: Fisher’s exact test, for categorical variables; Kruskal-Wallis test, for continuous variables.

    • ↵1 Mean dose 15.7 mg (SD 10.2).

    • ↵2 24/76 women (32%) and 18/39 men (46%) were anemic (hemoglobin < 115 g/l for women and 135 g/l for men. IQR: interquartile range; mRSS: modified Rodnan skin score; HAQ-DI: Health Assessment Questionnaire-Disability Index; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; DLCO: diffusion capacity for carbon monoxide.

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    Table 2.

    Effect of baseline confounders on baseline mRSS and change over time. Values are expressed as coefficient (95% CI).

    MeasurementEffect on Skin Score at Baseline, n = 751Effect on Skin Score Slope, n = 751
    Age, per 10 years*1.00 (0.07, 1.93)−0.83 (−1.48, −0.18)
    HAQ-DI score, per unit change3.32 (1.67, 4.97)−0.62 (−1.81, 0.57)
    Functional Questionnaire, per unit change0.31 (0.14, 0.49)−0.08 (−0.20, 0.04)
    Hemoglobin, per g/l−0.122 (−0.202, −0.041)0.028 (−0.0223, 0.079)
    Platelets, per 109/l0.022 (0.007, 0.036)0.005 (−0.006, 0.016)
    Plasma creatinine, per μmol/l0.007 (−0.009, 0.023)−0.015 (−0.022, −0.008)
    Pulmonary hypertension−0.05 (−3.97, 3.86)−1.88 (−7.34, 3.59)
    Pulmonary fibrosis−0.66 (−3.99, 2.68)−1.04 (−2.91, 0.82)
    Cardiac involvement0.18 (−4.30, 4.65)−1.26 (−4.56, 2.04)
    • ↵* Changes in skin score assessed per 10-year unit. HAQ-DI: Health Assessment Questionnaire-Disability Index.

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    Table 3.

    Changes in mRSS over time in the different treatment protocols. Values are expressed as coefficient (95% CI).

    Protocol 1, n = 29Protocol 2, n = 25Protocol 3, n = 61Protocol 4, n = 19p
    Present dataset−1.81 (−4.08, 0.460)−4.46 (−6.69, −2.23)−3.10 (−4.27, −1.93)−2.86 (−4.61, −1.11)0.43
    Present dataset (all subjects with weights ≥ 20 removed)n = 27 −0.786 (−3.55, 1.98)n = 23 −4.61 (−7.57, −1.65)n = 60 −3.49 (−4.76, −2.22)n = 16 −2.91 (−4.48, −1.34)0.28
    • mRSS: modified Rodnan skin score.

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1 Jan 2010
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Observational Study of Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis
ARIANE L. HERRICK, MARK LUNT, NINA WHIDBY, HOLLY ENNIS, ALAN SILMAN, NEIL McHUGH, CHRISTOPHER P. DENTON
The Journal of Rheumatology Jan 2010, 37 (1) 116-124; DOI: 10.3899/jrheum.090668

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Observational Study of Treatment Outcome in Early Diffuse Cutaneous Systemic Sclerosis
ARIANE L. HERRICK, MARK LUNT, NINA WHIDBY, HOLLY ENNIS, ALAN SILMAN, NEIL McHUGH, CHRISTOPHER P. DENTON
The Journal of Rheumatology Jan 2010, 37 (1) 116-124; DOI: 10.3899/jrheum.090668
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